Multiple System Atrophy Overview

Multiple system atrophy, or MSA, is a rare, degenerative neurologic condition that affects both men and women, usually starting in the 50’s or early 60’s1. MSA is considered a type of parkinsonism but with more widespread effects on the brain and body. The condition was first identified in 1962 and named Shy-Drager syndrome for two physicians who reported patients showing a combination of Parkinson-like movement disorders and problems with the autonomic, or body-regulating division of the nervous system2.

Common and Distinguishing Features

Similarities to Parkinsonism: Both Parkinson’s disease and MSA are characterized by deposits of a type of protein known as alpha-synuclein in the nervous system. Both conditions also specifically affect cells that produce dopamine,4, a neurotransmitter that controls motor commands. As a result, many of the same motor dysfunctions occur in the two conditions.

Unique Features: Important differences distinguish the symptoms and course of MSA from Parkinson’s disease and other conditions of the nervous system, such as cerebellar ataxia or pure autonomic failure (PAF). Notably, MSA affects several areas of the brain, including the cerebellum, your brain’s balance and coordination centers, and the autonomic nervous system, which controls your body’s automatic, or regulating functions, such as blood pressure, digestion and temperature.

Another distinguishing feature of MSA is the types of cells involved. While Parkinson’s disease affects the dopamine-producing neurons of a motor-controlling portion of the brain known as the nigro-striatial area, MSA affects both neurons and glial cells – support cells that maintain the health of neurons and which out number neurons by 10:15. Additionally, some of the glial cells affected in MSA produce myelin, the fatty substance that insulates neurons6.

Diagnosis

At this time there are no specific symptoms, blood tests or imaging studies that distinguish MSA. Instead, doctors rely on a combination of symptom history, physical examination and lab tests to evaluate the motor system, coordination and autonomic function to arrive at a probable diagnosis.

Despite the diagnostic challenge MSA poses, recent research has yielded promising results in ways that may help unravel the causes and detection of this disease. Medical technology such as functional MRI, or fMRI, shows activity levels in the brain and can demonstrate areas of impaired brain function9.

Additionally, by applying sensitive pattern recognition techniques to certain MRI studies, medical science is becoming increasingly better at teasing apart the early signs of MSA from Parkinson’s disease and other neurologic conditions with great accuracy10.

New studies are also finding that a particular type of lipid transporting molecule important for production of myelin might be faulty in MSA patients and that evaluating this molecule, known as ABCA8, could provide a causative explanation and a screening tool for MSA11.